Primary biliary cholangitis

Primary biliary cholangitis (previously referred to as primary biliary cirrhosis) is a chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1). The aetiology is not fully understood although it is thought to be an autoimmune condition. Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis. The classic presentation is itching in a middle-aged woman

Associations

• Sjogren's syndrome (seen in up to 80% of patients)
• rheumatoid arthritis
• systemic sclerosis
• thyroid disease

Clinical features

• early: may be asymptomatic (e.g. raised ALP on routine LFTs) or fatigue, pruritus
• cholestatic jaundice
• hyperpigmentation, especially over pressure points
• around 10% of patients have right upper quadrant pain
• xanthelasmas, xanthomata
• also: clubbing, hepatosplenomegaly
• late: may progress to liver failure

Diagnosis

• immunology
  • anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific
  • smooth muscle antibodies in 30% of patients
  • raised serum IgM